Lau Rheum

polymyalgia rheumatica aka PMR, polymyositis, reactive artheritis, RA, Sjogren syndrome, SLE, scleroderma

what is the SECRET acronym

describes clinical features of polymyalgia rheumatica

Stiffness and pain

Elderly (65+ yo)

Constitutional sx

Rheumatism (arthritis)

Elevated erythrocyte sedimentation rate

Temporal arteritis (giant cell arteritis)

temporal arteritis/giant cell arteritis can cause

blindness! no bueno

what is PMR

polymyalgia rheumatica

inflammatory syndrome characterized w/ pain and stiffness

who gets PMR

50+ yo, women 2x more than men

PMR dx criteria

pt is at least 50 yo

b/l sx of 2 out of three areas (neck, shoulder, hip) for at least 1 mo

ESR>40 mm/hr

exclusion of other dx

what is a unique feature of PMR

temporal arteritis

PMR onset

stiffness and pain usually insidious

shoulder often first to be affected

initially u/l then progresses to b/l

pain limiting mobility, dramatic stiffness/gelling, night pain awakening pt

PMR

PMR detectable synovitis

knee effusion, wrist synovitis, sternoclavicular synovitis

m/c synovitis in PMR

sternoclavicular

synovitis in PMR prognosis

usually in onset of dz, transient and relatively mild, rapid tapering from GCC therapy

wrist synovitis is usually associated w/

carpal tunnel syndrome

pt appears chronically ill, weight loss, fatigue, depression, low grade fever, neck/shoulder tenderness, nl muscle strength

PMR

what happens to muscle as PMR progresses

atrophy

where does joint movement cause pain in PMR pts and what causes it

in proximal extremities, not joints

proximal stiffness/pain caused by synovitis of shoulders and hips

PMR genetics

association w/ HLA-DR4

what kind of syndrome is PMR

chronic systemic inflammatory

PMR labs

elevated ESR (often >100), CRP, IL6

what is interleukin 6

cytokine that acts as a bridge between innate and adaptive immunity

involved in inflammation and immune responses

common heme findings in PMR

normochromic normocytic anemia, thrombocytosis

PMR liver enzymes

m/c is increased alk phos

PMR renal fxn, UA, serum CK

nl

PMR ANA and rheumatoid factor

negative

PMR synovial fluid testing

inflammatory w/ a poor mucin clot

PMR leukocytes

increased count w/ 40-50% polynorphonuclear leukocytes (PMNs)

when does temporal arteritis occur in PMR

can occur synchronously or sequentially

may be initial sx

current/recent HA, jaw claudication, visual disturbance, scalp tenderness

temporal arteritis

find temporal arteritis on PE

ascultate temporal pulse for bruits

fever, anemia, high ESR and/or CRP in pt 50+ yo

PMR

confirm PMR dx

biopsy of temporal artery

negative labs in PMR

CK, ANA, RF, anti cyclic citrullinated peptide, TSH

how do PMR pts die

giant cell arteritis, cerebrovascular events, MI, aortic aneurysm, aortic dissection

PMR tx goals

reduce sx, prevent vision loss

tx PMR

GCC, start w/ 40-60 mg/day for 1 mo then taper

35-65% get GCC toxicity

tx PMR ocular sx

methylprednisolone 1,000 mg/day for 3 days to protect remaining vision, then tx for 2 yrs

does NOT correct vision

monitoring during PMR tx

check ESR/CRP to monitor inflammatory dz activity (they should go down)

tx PMR giant cell arteritis

antil IL-6

162 mg SQ qwk or once every other week

combo w/ tapering course of GCC

tx PMR in pts w/ GCC toxicity and can’t tolerate tocilizumab

methotrexate

what shows better efficacy than GCC in PMR

abatacept

ASA in PMR

reduces cranial ischemic complications like stroke and scalp/tongue infarction

what kind of dz is polymyositis

inflammatory muscle dz, m/c of uncommon d/o

non suppurative (pus forming) muscle inflammation

polymyositis

polymyositis is m/c in

females, Black ppl

one peak at 10-15 yo, one at 45-55 yo

constitutional sx of polymyositis

fatigue, low grade fever, weight loss

MSK sx of polymyositis

arthralgia/arthritis

pulmonary sx of polymyositis

interstitial lung dz, aspiration PNA, respiratory muscle weakness, pulmonary HTN

GI sx of polymyositis

esophageal dysmotility, intestinal perforation from vasculitis

cardiac sx of polymyositis

dysrhythmias, tachycardia, a fib, AV blocks, bundle branch blocks, congestive HF

vascular sx of polymyositis

vasculitis, livedo reicularis, skin ulcerations, Raynaud’s phenomenon

what should you screen for in polymyositis

underlying neoplastic dz

hx and exam (breast/pelvic/prostate), stool occult blood, CXR, mammogram, routine labs

poor prognostic factors of polymyositis

ca, increased age, lung or cardiac involvement, late/previously inadequate tx

dx polymyositis

proximal muscle weakness in neck flexors, shoulder, pelvic girdle

elevated serum muscle enzyme (CK)

myoglobinemia and myoglobinuria (coca cola urine)

aspartate and alanine transferase from liver enzymes

lactate dehydrogenase

confirm dx of polymyositis

muscle biopsy shows perivascular inflammation w/ muscle fiber necrosis and muscle fiber regeneration

polymyositis is associated w/

connective tissue d/o, scleroderma, Sjogren’s syndrome, SLE, RA

polymyositis mainstay tx

CCS: prednisone started at 1-1.5 mg/kg/day until remission, then taper while monitoring for dz recurrence

polymyositis immunosuppresive agents

methotrexate, azathioprine, mycophenolate mofetil

tx polymyositis refractory to prednisone

IVIG to regulate immune system attacks on healthy muscles

polymyositis biologics

rituximab “for stubborn cases”

polymyositis rehab

PT, OT, speech therapy

reactive arthritis aka

Reiters syndrome

GI or genital infection leading to joint pain/swelling in knees ankles, conjunctivitis, rashes, urethritis

reactive arthritis (can’t see/pee/climb a tree)

reactive arthritis rheumatic dz category

seronegative spondyloarthritis

reactive arthritis genetics

associated w/ human leukocyte antigen HLA-B27

bacteria associated w/ reactive arthritis

generally enteric or venereal

Chlamydia trachomatis, Shigella flexneri, Salmonella typhimurium, Salmonella enteritidis

this dz can range from isolated transient monoarthritis or enthesitis to severe multisystem dz

reactive arthritis

reactive arthritis constitutional sx

common, include fatigue, malaise, fever, weight loss

reactive arthritis MSK sx

acute onset asymmetric painful arthritis primarily in knees, ankles, subtalar, metatarsophalangeal, toe interphalangeal

reactive arthritis urogenital lesions

men: urethritis and prostatitis

women: cervicitis or salpingitis

ocular reactive arthritis sx

conjunctivitis, anterior unveitis refractory to tx and leading to blindness

oral reactive arthritis sx

superficial, transient, painful ulcers

skin reactive arthritis sx

keratoderma blennorrhagica: vesicles and/or pustules which become hyperkeratonic, form crust then disappear, m/c on palms and soles

reactive arthritis penis

circinate balanitis

reactive arthritis pts benefit from high dose

NSAIDs

reactive arthritis get abx for

acute chlamydial urethritis, enteric infection

tx reactive arthritis C trachomatis or C pneumoniae

6 mo course of rifampin w/ azithromycin or doxycycline

tx reactive arthritis tendinitis and other enthesitic lesions

intralesional GCC

tx reactive arthritis uveitis

CCS drops/injection/oral combo w/ NSAID supportive mydratics (dilate pupil)

tx persistent reactive arthritis

immunosuppressants

reactive arthritis comprehensive management

counseling, PT, surveillance for long term complications

what is RA

chronic inflammatory dz of unknown etiology characterized by symmetric polyarthritis and m/c form of chronic inflammatory arthritis

systemic dz leading to extra articular manifestations including fatigue, SQ nodules, lung involvement, pericarditis, peripheral neuropathy, vasculitis, hematologic abnl

RA

dx RA

serum antibodies to anti citrullinated protein antibodies and RF

who has highest incidence of RA

25-55 yo, females

RA RF

smoking

RA pain is from inflammation of

joints, tendons, bursae

early morning stiffness in small joints of hands and feet that eases w/ physical activity

RA

RA joint involvement

can be mono/oligo/polyarticular in symmetric distribution

m/c joints are wrist, metacarpophalangeal, PIP

flexor tendon tenosynovitis is hallmark of

RA, leads to decreased ROM, reduced grip strength, trigger fingers

RA deformities

ulnar deviation, swan neck, boutonniere, z line, piano key movement, metarsophalangeal joint movement, pes planovalgus

RA ulnar deviation

from MCP sublux w/ partial dislocation

swan neck

seen in RA, hyperextension of PIP and flexion of DIP

boutonniere

seen in RA, PIP flexion and DIP hyperextension

z line deformity

seen in RA, first MCP sublux and hyperextension of first IP joint

piano key movement

seen in RA, inflammation at ulnar styloid and tenosynovitis of extensor carpi ulnaris which may cause distal ulna styloid sublux

MTP joint movement of feet

early feature of RA, chronic inflammation in feet is earliest, chronic inflammation of ankle and midtarsal comes later and causes pes planovalgus

RA constitutional sx

weight loss, fever, fatigue, malaise, depression, cachexia in severe cases

RA fever

101 F, should raise suspicion of systemic vasculitis or infection

primary site for RA inflammation

synovium: becomes boggy and edematous, develops villous projection

pannus

proliferative synovium in RA, leads to painful arthritis sx, may invade bone and cartilage and destroy joint

RA nodules

SQ w/ radiographic evidence of joint erosions

non tender, adherent to periosteum/tendons/bursae, develop in skeleton to repeated trauma/irritation